1/7. Testicular-sparing surgery for the prepubertal testicular tumor. Experience of two cases with large cell calcifying Sertoli cell tumors.PURPOSE: We review prepubertal germ cell tumors of testis in our institute and the Japanese registry and present 2 cases with a large cell calcifying sertoli cell tumor (LCCSCT) and discuss the possibility of testis-sparing surgery. MATERIALS AND methods: incidence, age, pathology and clinical stages of prepubertal germ cell tumors are surveyed for 30 years at our department and 10 years of the malignant tumor registry of the Japanese Society of Pediatric Surgery. Two representative prepubertal boys with LCCSCT are presented. One of them was treated by partial orchiectomy. RESULTS: The majority of testicular germ cell tumors in the prepubertal age were composed of embryonal cell carcinoma/yolk sac tumors or teratoma, occurred in preschool age, were limited to clinical stage I and did not metastasize irrespective of histology. Benign behavior which included recovery from hormonal derangement, no tumor recurrence and negative antisperm antigen was observed in 2 cases with LCCSCT who underwent either radical orchiectomy or partial orchiectomy. CONCLUSION: Partial orchiectomy should be considered as a standard option in prepubertal schoolboys with a testicular mass if surgically feasible. This surgical treatment is safe and preserves fertility and is psychologically advantageous. It is not recommended for yolk sac tumors that may recur, however they are rare in prepubertal boys and can be differentiated preoperatively by prudent evaluation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/7. High TGFbeta1, estrogen receptor, and aromatase gene expression in a large cell calcifying sertoli cell tumor (LCCSCT): implications for the mechanism of oncogenesis.Large cell calcifying Sertoli cell tumors (LCCSCT) are associated with carney complex and peutz-jeghers syndrome. The mechanisms linking these 2 genetic defects to the genesis of this tumor are obscure. Studies of CYP19 (aromatase) and transforming growth factor (TGF)-beta1 messenger rna (mRNA) abundance, estrogen receptor (ER), TGFbeta1, and TGFbeta type II receptor (R) immunochemistry were carried out in the testis of a patient with this tumor to gain information on possible mechanisms of cell tumor development. Testicular tissue of a prepubertal patient, collected at gonadectomy, was separated into 2 macroscopically distinct fractions: tumoral nodules (Tu) and extratumoral, normal-looking testicular tissue (ExTu). The patient was a 9.5-year-old boy with a 5-year history of bilateral gynecomastia (Tanner stage 4), no pubic hair, incipient genital development, and bilateral testicular nodules. Multiple pigmented lesions of the skin were present. Bilateral mammectomy and gonadectomy was performed. rna was extracted from Tu and ExTu for semiquantitative reverse transcriptase-polymerase chain reaction of CYP19 and TGFbeta1. Protein expression of ER, TGFbeta1, and TGFbeta type II R in Tu and ExTu was detected by immunohistochemistry. cell proliferation was estimated by ki-67 antigen immunochemistry and apoptosis using a modified TUNEL assay. Mean expression of aromatase and TGFbeta1 mRNAs in Tu was 6- and 2.3-fold higher than in ExTu, respectively (P<0.05). Tumoral cells exhibited ER staining with a predominant extranuclear localization. Positive staining of sertoli cells in Tu was higher than in ExTu. TGFbeta1 immunostaining of the interstitial cells in Tu was higher than in ExTu. TGFbeta type II R immunostaining was detected in most Sertoli and interstitial cells, but intensity in ExTu was lower than in Tu. No significant difference was detected in the proliferation index, but in Tu, the percentage of sertoli cells in apoptosis (1.4%) was significantly lower (P<0.01) than in ExTu (14.0%). The following hypothesis is proposed. The congenital gene defects of carney complex or of peutz-jeghers syndrome might trigger a cascade of intracellular events that leads to overexpression of aromatase in sertoli cells, favoring the development of a LCCSCT. At some point in the evolution of the disease, a mutational event might induce a higher expression of the ER. Also, TGFbeta1 protein expression is increased in neighboring cells. In this environment, TGFbeta1 might switch from tumor suppressor to oncogenic factor and, along with estrogen-ER complexes, might favor tumor progression by inhibiting apoptosis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/7. Localization of S-100 protein in a Leydig and Sertoli cell tumour of testis.A Sertoli-Leydig cell tumour of testis presented some diagnostic difficulties. The tumour cells showed strong expression of S-100 antigen. Preliminary study of non-neoplastic testis suggests that leydig cells and, to a lesser extent, sertoli cells express S-100 antigen; its localization may be of value in the diagnosis of sex cord-stromal tumours of testis.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
4/7. Ovarian sertoliform endometrioid carcinoma.Sertoliform endometrioid carcinoma (SEC) is a rare ovarian neoplasm occurring almost exclusively in post-menopausal patients. We studied a 71-year-old patient who underwent a total hysterectomy with bilateral salpingo-oophorectomy for a right ovarian mass measuring 25 cm in its maximal dimension. histology revealed an SEC, featuring foci of typical endometrioid carcinoma and areas of clear cell differentiation. This particular type of ovarian neoplasm, already described in 21 reported cases in the literature, must be distinguished from Sertoli cell tumours and Sertoli-Leydig cell tumours which are encountered at a younger age. We discuss the elements of the differential diagnosis and insist upon the value of anti-epithelial membrane antigen in identifying an SEC.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/7. A paradoxical inhibition of androgenic hyperproduction by a Sertoli-Leydig cell tumour ovary.A 17 year old woman was evaluated for amenorrhoea and severe hirsutism (Ferriman-Gallway index = 31). Pelvic ultrasound demonstrated a right unilateral ovarian mass (6 x 5 cm), whereas the computed tomography for the adrenal gland was normal. Endocrinological findings revealed normal concentrations of oestradiol, progesterone, dihydroepiandrosterone sulphate, cortisol, prolactin, follicle-stimulating hormone, luteinizing hormone and adrenocorticotrophic hormone (ACTH). Total testosterone, free testosterone, androstenedione and 17-hydroxy-progesterone concentrations, already elevated at basal conditions, did not increase after an ACTH test, whereas they decreased significantly after dexamethasone administration and increased after a human chorionic gonadotrophin test. Of all the tumour markers investigated, tissue polypeptide antigen and alpha 1-fetoprotein showed an increase in concentration. Selective venous ovarian catheterization indicated the presence of an androgen-producing tumour in the right ovary. The histopathological diagnosis was consistent with a Sertoli-Leydig cell tumour ranking between an intermediate and a poor grade of differentiation, with heterologous elements characterized by mucinous epithelium of the gastro-intestinal type. An endocrine evaluation performed postoperatively showed a normalization of all serum pathological hormones and tumour markers studied. Some particular aspects were focused on and discussed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/7. Sertoliform endometrial adenocarcinoma: a study of four cases.We studied four endometrial carcinomas with a conspicuous component that resembled patterns in Sertoli cell tumors. The patients presented at age 44-83 years (mean 65 years), with abnormal or postmenopausal vaginal bleeding in three and abnormal cervical cytology in one. All were multiparous, moderately to markedly obese, and hypertensive, and three patients had non-insulin-dependent diabetes mellitus. One tumor was suspected to be an endometrial stromal sarcoma with sex-cord-like differentiation on biopsy. Gross examination of the hysterectomy and bilateral salpingo-oophorectomy specimens showed solid polypoid endometrial tumors in each case. light microscopic examination showed three to be superficially invasive of the myometrium and one to be confined to the endometrium; none of the tumors showed the tongue-like pattern of myoinvasion or the angiolymphatic invasion characteristic of low-grade endometrial stromal sarcomas. The sertoliform component, which predominated in one case and was only focal in the three others, was composed of uniform small hollow tubules lined by columnar cells with apical cytoplasm and of compact slender cords. The tubules and cords were often present between benign-appearing or carcinomatous glands. In the case with predominate sertoliform areas, the lesional cells had clear cytoplasm suggesting a lipid-rich variant; special stains of this case demonstrated cytoplasmic glycogen but no fat. In none of the cases was cytoplasmic mucin, argyrophil granules, or argentaffinity demonstrated. The nonsertoliform areas of the tumors consisted of typical endometrioid adenocarcinoma; concurrent endometrial hyperplasia was also present in each case. Squamous differentiation and minor foci of anaplastic carcinoma with bizarre tumor giant cells were present in three tumors. Immunoperoxidase stains showed staining for two or more markers of epithelial or glandular differentiation in the sertoliform areas in all cases (keratin, epithelial membrane antigen, carcinoembryonic antigen, CA125, Tag72), with focal expression of vimentin in all cases. In none of the cases was desmin or actin staining observed. The evidence indicates that tumors in this series are variants of endometrioid adenocarcinoma and are distinct from uterine tumors resembling ovarian sex-cord tumors and stromal sarcomas with sex-cord-like differentiation.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
7/7. Testicular sertoli cell tumor with a heterologous sarcomatous component: immunohistochemical assessment of Sertoli cell differentiation.OBJECTIVE: Immunohistochemical staining is reported to be useful in distinguishing ovarian Sertoli-stromal cell tumors from carcinosarcomas. To assess Sertoli cell differentiation in a rare malignant biphasic testicular tumor, we compared the immunophenotypic profile of the tumor with that of Sertoli cell nodules and adenomas and mullerian carcinosarcomas. DESIGN: Immunohistochemical staining was performed on 6 testes (4 with hyperplastic Sertoli cell nodules, 2 with Sertoli cell adenomas) and 7 carcinosarcomas (6 involving the uterus, 1 involving the uterus and ovary) using primary monoclonal antibodies AE1/AE3, CAM 5.2, CA 19.9, and antibodies directed against epithelial membrane antigen, carcinoembryonic antigen (monoclonal and polyclonal), S100, placental alkaline phosphatase, and inhibin. These staining results were compared with those of the index case. RESULTS: All testes showed positive staining for inhibin and vimentin in the sertoli cells of the nodules and adenomas. One Sertoli cell nodule showed focal staining with AE1/AE3 and CAM 5.2. Both adenomas showed focal positive staining for S100. All nodules and adenomas were negative for epithelial membrane antigen, monoclonal and polyclonal carcinoembryonic antigen, CA 19.9, and placental alkaline phosphatase. In contrast, the carcinomatous areas of the carcinosarcomas were all negative for inhibin but exhibited positive staining for AE1/AE3, CAM 5.2, and epithelial membrane antigen. The carcinosarcomas showed variable expression of vimentin, S100, carcinoembryonic antigen, CA 19.9, and placental alkaline phosphatase. The epithelial component of the tumor from the index case showed strong diffuse staining for inhibin and vimentin and only very faint focal staining with AE1/AE3 and CAM 5.2. The epithelial component was negative for epithelial membrane antigen, monoclonal and polyclonal carcinoembryonic antigen, S100, CA 19.9, and placental alkaline phosphatase. CONCLUSIONS: The immunohistochemical findings in the index case support the diagnosis of sertoli cell tumor with a heterologous sarcomatous component over carcinosarcoma. Inhibin seems to be the best single marker for Sertoli cell differentiation. To our knowledge, only 1 other case of this rare testicular tumor has been reported in the literature.- - - - - - - - - - ranking = 8keywords = antigen (Clic here for more details about this article) |